Ebook Pediatric palliative care: Part 1

Pediatric
Palliative Care
Edited by

Stefan J. Friedrichsdorf
Printed Edition of the Special Issue Published in Children


Pediatric Palliative Care



Pediatric Palliative Care

Special Issue Editor
Stefan J. Friedrichsdorf

MDPI • Basel • Beijing • Wuhan • Barcelona • Belgrade


Special Issue Editor
Stefan J. Friedrichsdorf, MD, FAAP
Department of Pain Medicine, Palliative Care & Integrative Medicine,
Children’s Hospitals and Clinics of Minnesota
Minneapolis, Minnesota, USA

Editorial Office
MDPI
St. Alban-Anlage 66
4052 Basel, Switzerland

This is a reprint of articles from the Special Issue published online in the open access journal Children
(ISSN 2227-9067) from 2017 to 2018 (available at: />issues/palliative care)

For citation purposes, cite each article independently as indicated on the article page online and as
indicated below:
LastName, A.A.; LastName, B.B.; LastName, C.C. Article Title. Journal Name Year, Article Number,
Page Range.

ISBN 978-3-03897-350-8 (Pbk)
ISBN 978-3-03897-351-5 (PDF)

Cover image courtesy of a boy with serious illness: ”PAIN. Once I felt as if an I.V. was exploding in
my arm!”
The child then described the excruciating pain he had felt when someone tripped over his IV pole that then came
crashing down. The boldly colored, nightmarish image with foreboding slashes of black conveys the extreme
pain and the associated anxiety. and vulnerability. At the bottom of the picture—totally overwhelmed by the
chaos—is his arm, the site of the pain.

Articles in this volume are Open Access and distributed under the Creative Commons Attribution
(CC BY) license, which allows users to download, copy and build upon published articles even for
commercial purposes, as long as the author and publisher are properly credited, which ensures
maximum dissemination and a wider impact of our publications. The book taken as a whole
is c 2019 MDPI, Basel, Switzerland, distributed under the terms and conditions of the Creative
Commons license CC BY-NC-ND ( />

Contents
About the Special Issue Editor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii
Stefan J. Friedrichsdorf and Eduardo Bruera
Delivering Pediatric Palliative Care: From Denial, Palliphobia, Pallilalia to Palliactive
Reprinted from: Children 2018, 5, 120, doi:10.3390/children5090120 . . . . . . . . . . . . . . . . .

1

Brian S. Carter
Pediatric Palliative Care in Infants and Neonates
Reprinted from: Children 2018, 5, 21, doi:10.3390/children5020021 . . . . . . . . . . . . . . . . . . 14
Harold Siden
Pediatric Palliative Care for Children with Progressive Non-Malignant Diseases
Reprinted from: Children 2018, 5, 28, doi:10.3390/children5020028 . . . . . . . . . . . . . . . . . . 23
Julie Hauer
Feeding Intolerance in Children with Severe Impairment of the Central Nervous System:
Strategies for Treatment and Prevention
Reprinted from: Children 2018, 5, 1, doi:10.3390/children5010001 . . . . . . . . . . . . . . . . . . . 32
Timothy A. Warlow and Richard D.W. Hain
‘Total Pain’ in Children with Severe Neurological Impairment
Reprinted from: Children 2018, 5, 13, doi:10.3390/children5010013 . . . . . . . . . . . . . . . . . . 43
Julia Downing, Sue Boucher, Alex Daniels and Busi Nkosi
Paediatric Palliative Care in Resource-Poor Countries
Reprinted from: Children 2018, 5, 27, doi:10.3390/children5020027 . . . . . . . . . . . . . . . . . . 53
Ross Drake
Palliative Care for Children in Hospital: Essential Roles
Reprinted from: Children 2018, 5, 26, doi:10.3390/children5020026 . . . . . . . . . . . . . . . . . . 65
Martha F. Mherekumombe
From Inpatient to Clinic to Home to Hospice and Back: Using the “Pop Up” Pediatric Palliative
Model of Care
Reprinted from: Children 2018, 5, 55, doi:10.3390/children5050055 . . . . . . . . . . . . . . . . . . 71
Andrea Postier, Kris Catrine and Stacy Remke
Interdisciplinary Pediatric Palliative Care Team Involvement in Compassionate Extubation at
Home: From Shared Decision-Making to Bereavement
Reprinted from: Children 2018, 5, 37, doi:10.3390/children5030037 . . . . . . . . . . . . . . . . . . 77

Kate Shafto, Suzanne Gouda, Kris Catrine and Melanie L. Brown
Integrative Approaches in Pediatric Palliative Care
Reprinted from: Children 2018, 5, 75, doi:10.3390/children5060075 . . . . . . . . . . . . . . . . . . 89
Lindsay J. Blazin, Cherilyn Cecchini, Catherine Habashy, Erica C. Kaye and Justin N. Baker
Communicating Effectively in Pediatric Cancer Care: Translating Evidence into Practice
Reprinted from: Children 2018, 5, 40, doi:10.3390/children5030040 . . . . . . . . . . . . . . . . . . 101
Kendra D. Koch and Barbara L. Jones
Supporting Parent Caregivers of Children with Life-Limiting Illness
Reprinted from: Children 2018, 5, 85, doi:10.3390/children5070085 . . . . . . . . . . . . . . . . . . 117
v


Barbara M. Sourkes
Children’s Experience of Symptoms: Narratives through Words and Images †
Reprinted from: Children 2018, 5, 53, doi:10.3390/children5040053 . . . . . . . . . . . . . . . . . . 135
Katherine E. Nelson, James A. Feinstein, Cynthia A. Gerhardt, Abby R. Rosenberg,
Kimberley Widger, Jennifer A. Faerber and Chris Feudtner
Emerging Methodologies in Pediatric Palliative Care Research: Six Case Studies
Reprinted from: Children 2018, 5, 32, doi:10.3390/children5030032 . . . . . . . . . . . . . . . . . . 148
Katharine E. Brock, Joanne Wolfe and Christina Ullrich
From the Child’s Word to Clinical Intervention: Novel, New, and Innovative Approaches to
Symptoms in Pediatric Palliative Care
Reprinted from: Children 2018, 5, 45, doi:10.3390/children5040045 . . . . . . . . . . . . . . . . . . 161
Tiina Jaaniste, Sandra Coombs, Theresa J. Donnelly, Norm Kelk and Danielle Beston
Risk and Resilience Factors Related to Parental Bereavement Following the Death of a Child
with a Life-Limiting Condition
Reprinted from: Children 2017, 4, 96, doi:10.3390/children4110096 . . . . . . . . . . . . . . . . . . 177
Meaghann S. Weaver, Cheryl Darnall, Sue Bace, Catherine Vail, Andrew MacFadyen and
Christopher Wichman
Trending Longitudinal Agreement between Parent and Child Perceptions of Quality of Life for

Pediatric Palliative Care Patients
Reprinted from: Children 2017, 4, 65, doi:10.3390/children4080065 . . . . . . . . . . . . . . . . . . 199
Sara Munoz-Blanco,
˜
Jessica C. Raisanen, Pamela K. Donohue and Renee D. Boss
Enhancing Pediatric Palliative Care for Latino Children and Their Families: A Review of the
Literature and Recommendations for Research and Practice in the United States
Reprinted from: Children 2018, 5, 2, doi:10.3390/children5010002 . . . . . . . . . . . . . . . . . . . 205
Jean Kelly, Jo Ritchie, Leigh Donovan, Carol Graham and Anthony Herbert
A Retrospective Review of Resuscitation Planning at a Children’s Hospital
Reprinted from: Children 2018, 5, 9, doi:10.3390/children5010009 . . . . . . . . . . . . . . . . . . . 217
Larissa Alice Dreier, Boris Zernikow, Markus Blankenburg and Julia Wager
A Sleep Questionnaire for Children with Severe Psychomotor Impairment
(SNAKE)—Concordance with a Global Rating of Sleep Quality
Reprinted from: Children 2018, 5, 20, doi:10.3390/children5020020 . . . . . . . . . . . . . . . . . . 229
Taelyr Weekly, Nicole Walker, Jill Beck, Sean Akers and Meaghann Weaver
A Review of Apps for Calming, Relaxation, and Mindfulness Interventions for Pediatric
Palliative Care Patients
Reprinted from: Children 2018, 5, 16, doi:10.3390/children5020016 . . . . . . . . . . . . . . . . . . 237

vi


About the Special Issue Editor
Stefan J. Friedrichsdorf, MD, FAAP is an Asssociate Professor of Pediatrics at the University of
Minnesota and the medical director of the Department of Pain Medicine, Palliative Care and
Integrative Medicine at Children’s Hospitals and Clinics in Minnesota, Minneapolis/St. Paul (USA)
—one of the largest and most comprehensive programs of its kind in the country. He is the presidentelect of the Special Interest Group on Pain in Childhood of the International Association for the Study
of Pain (IASP). The interdisciplinary pain program is devoted to prevent and treat acute, procedural,
neuropathic, psycho-social-spiritual, visceral, and persistent pain for all pediatric patients in close

collaboration with all subspecialties at Children’s Minnesota. The palliative care team provides
holistic care for pediatric patients with life-limiting diseases and adds an extra layer of support to the
care of children with serious illness and their families. Integrative Medicine provides and teaches
integrative,

non-pharmacological

therapies

(such

as

massage,

acupuncture/acupressure,

biofeedback, aromatherapy, self-hypnosis) to provide care that promotes optimal health and
supports the highest level of functioning in all individual child’s activities. Dr. Friedrichsdorf sees
pediatric patients as inpatients, in the interdisciplinary pain clinic, or in the palliative care clinic. He
is the sponsor of the system-wide lean value stream “The Comfort Promise: We do everything
possible to prevent and treat pain” at Children’s Minnesota to minimize and prevent pain caused by
needles for all in- and outpatients, reaching more than 200,000 children annually. In 2015 the
department opened an award winning, unique 10,000 square feet “Healing Environment” Pain,
Palliative and Integrative Medicine Clinic. Dr. Friedrichsdorf received the 2016 “Elizabeth Narcessian
Award for Outstanding Educational Achievements in the Field of Pain” by the American Pain Society
and the 2011 “Hastings Center Cunniff-Dixon Physician Award”. The department received the
“Circle of Life Award” by the American Hospital Association in 2008, was selected as a Palliative
Care Leadership Center for the Center to Advance Palliative Care (CAPC) 2008-2015, and the 2013
recipient of the “Clinical Centers of Excellence in Pain Management Award” by the American Pain

Society. In 2016 Children’s Minnesota received the prestigious ChildKind International pain relief
certification. He is associate editor of the Journal of Pain and Symptom Management, the principal
investigator of a 2010–2017 National Institutes of Health (NIH) / National Cancer Institute (NCI)
multisite study on the creation, implementation and evaluation of a Pediatric Palliative Care
Curriculum (EPEC-Pediatrics) and he founded and continues to direct the annual Pediatric Pain
Master Class, a week-long intensive interdisciplinary course for health professionals. Dr.
Friedrichsdorf has presented more than 650 lectures about pediatric pain medicine and palliative care
in 28 countries on all six continents and has a track record of research and publications in the field,
including contributions to more than 20 books on the subject. Dr. Friedrichsdorf received his MD
degree from the Medical University of L ubeck,
¨
Germany, completed his pediatric residency at the
University of Witten/Herdecke, Germany (Children’s Hospital in Datteln), and undertook his
fellowship in Pediatric Pain & Palliative Care at the University of Sydney, Australia (Children’s
Hospital at Westmead). He is board certified in Pediatrics, a Fellow of the American Academy of
Pediatrics and trained in hypnosis.

vii



children
Editorial

Delivering Pediatric Palliative Care: From Denial,
Palliphobia, Pallilalia to Palliactive
Stefan J. Friedrichsdorf 1,2, * and Eduardo Bruera 3
1
2
3

*

Children’s Hospitals and Clinics of Minnesota, 2525 Chicago Ave S, Minneapolis, MN 55403, USA
University of Minnesota Medical School, 420 Delaware Street SE, Minneapolis, MN 55455, USA
Department of Palliative Care and Rehabilitation Medicine, The University of Texas,
MD Anderson Cancer Center, 1515 Holcombe Blvd., Houston, TX 77030, USA;
Correspondence: ; Tel.: +1-612-813-7888; Fax: +1-612-813-7199

Received: 23 August 2018; Accepted: 28 August 2018; Published: 31 August 2018

Abstract: Among the over 21 million children with life-limiting conditions worldwide that would
benefit annually from a pediatric palliative care (PPC) approach, more than eight million would
need specialized PPC services. In the United States alone, more than 42,000 children die every year,
half of them infants younger than one year. Advanced interdisciplinary pediatric palliative care for
children with serious illnesses is now an expected standard of pediatric medicine. Unfortunately,
in many institutions there remain significant barriers to achieving optimal care related to lack of
formal education, reimbursement issues, the emotional impact of caring for a dying child, and most
importantly, the lack of interdisciplinary PPC teams with sufficient staffing and funding. Data reveals
the majority of distressing symptoms in children with serious illness (such as pain, dyspnea and
nausea/vomiting) were not addressed during their end-of-life period, and when treated, therapy
was commonly ineffective. Whenever possible, treatment should focus on continued efforts to
control the underlying illness. At the same time, children and their families should have access to
interdisciplinary care aimed at promoting optimal physical, psychological and spiritual wellbeing.
Persistent myths and misconceptions have led to inadequate symptom control in children with
life-limiting diseases. Pediatric Palliative Care advocates the provision of comfort care, pain,
and symptom management concurrently with disease-directed treatments. Families no longer have
to opt for one over the other. They can pursue both, and include integrative care to maximize the
child’s quality of life. Since most of the sickest children with serious illness are being taken care of
in a hospital, every children’s hospital is now expected to offer an interdisciplinary palliative care

service as the standard of care. This article addresses common myths and misconceptions which may
pose clinical obstacles to effective PPC delivery and discusses the four typical stages of pediatric
palliative care program implementation.
Keywords: pediatric palliative care;
program implementation

program development;

barriers;

hospice;

myths;

1. Introduction
The special edition “Pediatric Palliative Care” in Children ( />children/special_issues/palliative_care) has collated 20 outstanding articles from many of the leading
pediatric palliative care researchers and clinicians worldwide allowing us to present an overview of
advances, research, and challenges in pediatric palliative care (PPC). As the guest editor, I thank the
authors for their strong contributions to this edition, in assisting children with serious illness and their
families, as well as to moving our field further along.
Over 21 million children 0–19 years would benefit annually from a palliative care approach
worldwide, more than eight million needing specialized PPC [1,2]. In the United States alone, more than
Children 2018, 5, 120; doi:10.3390/children5090120

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Children 2018, 5, 120

42,000 children died in 2013, fifty-five percent of them infants younger than one year [3]. The leading
causes of pediatric deaths include accidents (7645 children), suicide (2143), and homicide (2021).
Leading life-limiting conditions include congenital malformations and chromosomal abnormalities
(5740) followed by malignancies (1850). Minorities, such as Latinos, appear to have higher barriers to
accessing PPC [4].
PPC is about matching treatment to patient goals and is considered specialized medical care
for children with serious illness. It is focused on relieving pain, distressing symptoms, and stress
of a serious illness and appropriate at any age and at any stage, together with curative treatment.
The primary goal is to improve quality of life for both the child and his or her family. In the words of an
ill child: “Palliative care no longer means helping children die well, it means helping children and their
families to live well, and then, when the time is certain, to help them die gently.” (Mattie Stepanek,
1990–2007). Sadly, advances in the control of symptoms in children dying of diseases such as cancer
have not kept pace with treatment directed at curing the underlying disease. The majority of distressing
symptoms in children with advanced cancer (such as pain, dyspnea and nausea/vomiting) were not
treated, and when treated, therapy was commonly ineffective [5–9].
In this editorial we will address the four steps of PPC program implementation [10], followed by
an evaluation of common assumptions, myths and barriers, which may hinder the implementation of
PPC into the care of a child or teenager with serious illnesses [11,12].
2. Implementing Institutional Change: The Four Stages of PPC Program Implementation
Although the majority of children’s hospitals in the United States do have a pediatric palliative
care program, most of them appear to be understaffed and underfunded. A survey by Feudtner et al. in
2013 among 226 US children’s hospitals (of which 162 responded) showed that 112 (69% of respondents;
at least 49.6% of all children’s hospitals) have PPC program [13]. However, most programs offer only
inpatient services, and most only during the work week.
A universal reality is that, overwhelmingly, in places where palliative care has not existed before,
will require major cultural adaptation [10]. We have adapted the following four stages, initially
described by Bruera in 2004 [10], to pediatric palliative care program implementation.
2.1. Stage 1: Denial
Clinical leaders suggesting the implementation of a new PPC program in a pediatric institution

are very likely to face significant denial by clinical colleagues and hospital executives, who may not be
aware of the need for a PPC program. Often, there are limited or no measurement of the amount of
physical and emotional distress suffered by children and their families with serious illness. The limited
documentation on the need of PPC is complicated by perceptions (which are unfortunately not based
on reality) such as “Our patients here usually have very good symptom control” or “We here at . . . ”
(insert the name of any pediatric primary service or subspecialty here) “ . . . already got it and we
cover everything and don’t need an extra palliative care service.”
An important approach to expect and address denial, would be to carefully and rapidly document
the level of unmet need in patients and families in the pediatric institution, including in the patients
under care by individuals, who are in denial. Simple surveys of uncontrolled symptoms or emotional
distress might be extremely useful. Parents’ testaments, in writing or in a short movie clip, might be
particularly helpful to overcome denial during a presentation to colleagues and leadership.
2.2. Stage 2: Palliphobia
The second stage of PPC program implementation in a pediatric institution can be best described
as the recognition that there is a problem, but usually this meets consistent fear about consequences of
the problem and the possible solutions it entails. Although this might represent fear of the unknown,
it is not unheard of that sometimes people react with great anger towards it. In fact, many individuals
can react in negative way to developing a PPC team. Physicians, nurse practitioners and other health
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Children 2018, 5, 120

professionals may feel their professional competence is being questioned or even threatened by the
new PPC team. In fact, they may be afraid of referring patients to the PPC service because of concerns
over criticism of their symptom control, communication or overall treatment strategies they have
used for many years. Hospitals executives may be afraid of being found lacking in compassion and
holistic care among all the competing priorities in a shifting landscape of pediatric healthcare and
scarce funding.
Common phrases PPC teams may hear include “The parents are not ready”, “We asked them and

they said no” (although surprisingly few parents are asked for permission to include other services,
such as infectious disease services),“She is not dying now”, “There is always something else we can
try”, “It is too early”, “Hospice? That’s wonderful, but that is for other people”, “You talking to the
family means destroying hope”, “We are still fighting”.
Unfortunately, further exacerbation of Palliphobia can be expected, if the PPC team tries too hard
and/or is too fast in making changes in patient care. After a major confrontation with the neonatologist,
oncologist etc. (a.k.a. the bull-in-the-china-shop-strategy—“move aside, the palliative care doctor
has arrived . . . ”), the PPC usually succeeds in changing and improving analgesia, communication
strategies or changing discharge planning, but likely will not receive further consults/referrals from
this team or unit, resulting in a negative reputation among colleagues in the institution.
A useful technique is to approach a limited number of possible referring clinicians and ask them
to become supporters and mentors of the program. Once a significant number of patients have been
treated, it is then safe to make a presentation in Grand rounds or Medical staff meetings showing the
results, ideally with the presence of the referring clinicians. In absence of data, all opinions (including
palliphobic ones) are good, but the presence of data on positive outcomes and testimonials from
the initial team of referring clinicians can be very reassuring to those who are still uncommitted to
refer patients.
“Palliphobia” is more difficult to overcome than “Denial”, and requires disciplined planning and
rapid conflict resolution: Useful strategies to overcome this stage may include making great efforts at
reassuring the existing clinical pediatric team that the PPC of course will work in an integrated fashion
collaboratively with them. Importantly, members of the PPC team will not disqualify their patient
care plan, but rather enhance them by focusing on aspects not addressed so far. “How can I help?”
is commonly a useful question posed to the primary clinician, followed by a detailed discussion of
who does what (“Would you like me to prescribe the methadone, or do you prefer me simply giving
recommendations and you take care of it?”).
2.3. Stage 3: Pallilalia
A large number of PPC programs worldwide appear to be stuck in the 3rd stage “Pallilalia”.
Usually about two to four years after implementing a PPC program, repetitive absurdities are spoken
about palliative care in general and the PPC program specifically, without anything being done to
advance its development. This is in fact the most dangerous stage, as it has a high risk resulting in

burnout among PPC professionals.
Hospital leadership and executives describe PPC as “very important” or “a major priority”,
but actually there’s no significant allocation of personnel, space, money, curriculum time etc.
Colleagues within the pediatric institution frequently talk about how useful they feel PPC is,
and how happy they are with the PPC team, but as a matter of fact refer only small minority of
their patients—usually the ones with terrible psycho-social-medical and/or mental health problems.
With this attitude, a PPC program is simply financially and administratively not viable.
Children’s hospitals at this stage frequently “appoint a committee to discuss PPC”, or propose
“a major study whether PPC works in our institution” or, suggest the PPC lead applies for external
grants, so “funds can be obtained for a pilot program in a year or two”. There have also been cases,
where hospital leadership decided that the 6-figure donation to the PPC program were used to offset
general losses in other departments instead.

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Children 2018, 5, 120

Most understaffed PPC programs (commonly lead by a 0.2 FTE physician, who was on-call 24/7
and during his/her vacations), have disappeared at this stage. Clinical colleagues and institutions
became used to the beneficial presence of the PPC team, while having made no major commitment to
support it.
A useful technique to overcome Pallilalia is to use benchmarks for clinical and time burden
that might allow for fair comparisons. While some of the referring teams may see a mix of more
and less time demanding encounters, palliative care teams always see time demanding patients and
they frequently allow the referring teams to shorten their own encounter with the patients they refer.
Therefore, clinical time may be a better measure than number of encounters. Since this is not always
easy to measure, one palliative care team compared data from parking records to determine the
additional burden on the palliative care team.
It is important to anticipate this developmental stage by gathering data of provided clinical

services, number of patients seen, details of teaching conducted, research studies, etc. The PPC team
should aim mostly at leaders of hospital and medical schools, rather than just immediate supervisors or
peers, when providing documentation of work. They should request, that information be compared to
output and resources of other programs within the institution and on a national level. PPC teams may
ask for external review by national or international PPC leaders in the field. Sadly, not infrequently,
only through resignation of the PPC leader, institutions come to realize that PPC has been badly
under-resourced, and start corrective measures during the process of recruiting a successor.
2.4. Stage 4: Palliactive
A PPC program has reached the final level of development, when recognized by robust
appointment and funding of professional interdisciplinary team PPC members. It is recommended to
seek a designation of an administrative structure equal to oncology or infectious disease programs
(department or division). Other important components of this stage include allocation of space, formal
curriculum space and medical training program rotations.
PPC is truly recognized when colleagues actively refer patients to PPC and encourage other
colleagues to do the same. Unfortunately, regression can happen at any time—frequently due to
changes in administrative leadership within the institution. Also, in large children’s hospitals on
certain units is not unlikely that a robust established PPC team may encounter all four stages on the
very same day, depending on which physician they encounter.
3. Common Myths and Misconceptions in Pediatric Palliative Care (PPC)
3.1. Myth 1: PPC Is Primarily for Children with a Malignancy
The majority of children dying of serious illness do not have cancer [14]. In 2013 a total
of 42,328 children 0–19 years died in the United States, more than 55% (23,440) of them infants
younger than one year [3]. The leading causes of pediatric deaths include accidents (7645 children),
suicide (2143), and homicide (2021). In 2015 a total of 11,933 children, adolescents and young adults
0–24 years died due to a life-limiting disease: Leading conditions include congenital malformations
and chromosomal abnormalities (5965), followed by malignancies (2688), and heart disease (1354) [15].
Although the prognosis for children with cancer has improved considerably over the last decades,
malignancies now remains the leading cause of non-accidental death in childhood only in children older
than one year of age. In pediatric cancer units, the presence of “trigger diagnoses” (triggering automatic
referral to PPC) increased likelihood of palliative principle introduction 3.4 times (p < 0.003) [16].

That said, when providing interdisciplinary PPC services for children, it can be expected that
most children do not have a malignancy [17] and about half of the patients would be infants. Despite
the great need in neonatology, more than 45% of institution in Canada and the United States not have
neonatal comfort care guidelines, and of those reporting institutional neonatal comfort care guidelines,
19.1% do not address pain symptom management [18]. More than 90% of respondents in the same

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Children 2018, 5, 120

study felt that their institution would benefit from further education and training in neonatal EOL
care. Carter elaborates on PPC for babies in his article [19] in this special edition.
3.2. Myth 2: PPC Begins when Curative Treatments Stop
Sometimes clinicians, patients and families incorrectly assume that PPC is only appropriate when
all curative treatments are exhausted and discontinued and/or when a child is close to dying. In fact,
PPC is recommended to commence at diagnosis of a life-threatening disease, to continue through the
trajectory of the illness, and does not equal end-of-life care (but certainly includes it). PPC services
extend beyond the child’s death to the family during bereavement [20]. Earlier recognition by both
physicians and parents that the child had no realistic chance of cure led to a stronger emphasis on
treatment to lessen suffering and integrate PPC in pediatric cancer patients [21].
The overall improvement in the prognosis of serious illnesses, and the emotional involvement
in trying to save the life of a child may prevent both physicians and parents from discontinuing
therapies. The pursuit of such therapy modalities may overshadow attention to advanced prevention
and control of distressing symptoms and to quality of life and, which unfortunately may result in
increased suffering during child’s end-of-life period. However, it is sometimes not possible for parents
and/or the child to forgo further disease-directed treatment, and this should not be required in order
to achieve optimal palliative care. The need to ensure that everything possible has been done may be
the only way that some parents can live and cope with their child’s death [22].
The 2010 “Concurrent Care for Children” requirement of the United States Affordable Care Act

has this at heart: The 2016 briefing of the Mary J. Labyak Institute for Innovation at the National Center
for Care at the End of Life [23] described concurrent care for children with serious illness as follows:
“Until 2010, parents in all but a few states in the United States were faced with forgoing curative
treatments for their children to be eligible for hospice services. Or conversely, they were not eligible for
beneficial interdisciplinary hospice services while getting curative treatment. The Patient Protection
and Affordable Care Act (ACA) changed that situation. It requires all state Medicaid programs to
pay for both curative and hospice services for children under age 21 who qualify. On 23 March 2010,
President Obama signed ACA into law enacting a new provision, Section 2302, termed the “Concurrent
Care for Children” Requirement (CCCR). Section 2302 states that a child who is eligible for and receives
hospice care must also have all other services provided, or have payment made for, services that are
related to the treatment of the child’s condition.1 This provision affects children who are eligible for
Medicaid or the Children’s Health Insurance Program (CHIP). In its simplest form, implementation of
this provision could be accomplished by the state Medicaid agency eliminating any provider claims
that deny or delay concurrent curative care and hospice claims [23].”
Pediatric oncology providers in a recent survey [24] issued a highly favorable opinion about their
institution’s PPC service and agreed that early consultation is ideal. However, they report formally
consulting PPC is extremely difficult because of what the PPC symbolizes to families and the emotional
labor that the oncology provider must manage in introducing them.
3.3. Myth 3: Pediatric Palliative Care Involvement Shortens Life
Clinicians trained in palliative care will never issue a statement such as “There is nothing else
we can do”. Quite the opposite, advanced PPC teams may say “there is always a lot we can do”.
Even when the underlying life-limiting disease cannot be cured, sophisticated medical technology will
be utilized to improve the quality of life of the child and his and her family and to prevent and treat
distressing symptom. As such, PPC is therefore a very active and advanced approach to symptom
management and family support.
It appears, that a palliative care consult for patients with serious illness is associated with longer
survival and better quality of life: In the ENABLE III study, patients who received early palliative
oncology care had significantly longer 1-year survival rates than those who received delayed palliative
care [25]. In another innovative randomized controlled trial adults with advanced lung cancer receiving

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Children 2018, 5, 120

a palliative care intervention (providing appropriate and beneficial treatments) at the point of diagnosis,
in fact increased the quality of life, decreased depression, and led to a prolonged life (11.6 months vs.
8.9 months) [26]. These results underscore the need for palliative care early in a serious illness and
refute the notion that palliative care means giving up. Patients received palliative care alongside their
curative treatment. There is now emerging evidence that the inclusion of PPC specialists improves the
outcome of children with advanced serious illnesses, and sometimes represents the means to allows for
curative care through advanced symptom management provided by PPC [27]. Children who received
pediatric palliative home care were more likely to have fun (70% versus 45%) and to experience events
that added meaning to life (89% versus 63%) [9]. In addition, families who received PPC services report
improved communication [28] and children receiving PPC experience shorter hospitalizations and
fewer emergency department visits [29]. Parents of children with cancer who received PPC reported
less distress from pain, dyspnea and anxiety during the end-of-life period [8]. Undertaking research in
PPC is inherently difficult, and Nelson et al. in this special issue address this in their article [30].
The advanced pain and symptom management may explain the increase of survival in patients
with serious illness enrolled into PPC. Brock et al. describe in this special edition describe emerging
methods of symptom and health-related quality-of-life assessment through patient-reported outcomes
tools [31]. Data has shown, that distressing pain is very common among inpatients referred to palliative
care and three-quarters of patients with pain improve and improvement in pain is associated with
other symptom improvement [32]. The involvement of PPC team with adolescent and young adult
oncology patients is associated with the receipt of less intensive treatments during the last month of
life, such as being on a ventilator, invasive procedures, and fewer deaths in the intensive care unit [33].
Advanced pain management for children with serious illness often requires multimodal analgesia.
This describes an approach of utilizing multiple analgesic agents (such as basic analgesia, opioids,
adjuvant analgesia), regional anesthesia (such as nerve blocks or neuroaxial analgesia), rehabilitation
(such as physical therapy, motor graded imagery), psychological (such as cognitive behavioral therapy)

and integrative (formally known as “non-pharmacological”) therapies (such as massage, hypnosis)
which usually act synergistically for more effective pediatric pain control with fewer side effects than
a single analgesic or modality [34–37].
Harlow and Hain [38] explore the concept of total pain in the largest group of children with
life-limiting diseases, pediatric patients with severe neurological impairment.
3.4. Myth 4: PPC Can Only Be Provided Comprehensively within a Children’s Hospital
Currently most children in resource rich countries die in a hospital, most commonly in an intensive
care unit. In a recent study [39] reviewing all pediatric intensive care unit (PICU) admissions over
15 months of 89,127 children in the United Kingdom, children with life-limiting conditions constituted
57.6% of all admissions. Of the 4821 children who died on the PICU during that period, 72.9% had
a life-limiting condition. Since most of the sickest children with serious illness are being taken care
of in a hospital, every children’s hospital is now expected to offer an interdisciplinary palliative care
service as the standard of care [40].
One of the early PPC pioneers, Dr. Ann Goldman from the Department of Hematology/Oncology
at Great Ormond Street in London, UK implemented a “Symptom Care Team,” a team of nurses,
who were introduced at cancer diagnosis to the child and family. All children received home
visits after their first discharge. Children with high-risk cancer or relapses then already knew the
“Symptom Care Team”, which provided a 24/7 service, from the time of diagnosis. Between 1978–1981,
before the implementation of the “Symptom Care Team” only 19% of children with cancer died at
home. Then, between 1989–1990, after implementation of the team 77% of the children dying from
malignancies did so at home [41].
The death of a child with a serious illness at home may promote better family adjustment and
healing [42,43]. This could be related to decrease helplessness and increased family intimacy by being
at home. On the other hand, some have reported that family relationships appeared to be better when

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the child died in the hospital [44]. While it is often suggested that most children prefer to die at home,
this has actually not been systematically evaluated.
Parents of terminally ill children often wish for home care [45,46], and there is a not surprisingly
a positive correlation between availability of palliative home care and the number of children dying at
home [9,47–49]. The interdisciplinary PPC Team involvement in compassionate extubation at home
has been explored by Postier et al. in this special issue [50]. Most families regard caring for their
dying child as a positive experience [45]. It is critically important to discuss preferences regarding the
primary location of care as early as possible. A parental decision to care for their terminally ill child at
home involves consideration of medical, psychological, social and cultural factors together with such
practical considerations as the availability of respite care, physician access, and financial resources.
Children with advanced cancer who also received PPC home care or hospice were significantly more
likely to have fun (70% versus 45%), experience events that added meaning to life (89% versus 63%),
and to die at home (93% versus 20%) [9]. Whatever the decision is regarding the primary location
of care, families should be reassured that they can change from one option to another and that the
primary team will remain closely involved [45].
There appears to be a growing consensus of most pediatric palliative care specialists, that advanced
PPC for children with serious illness needs to be offered and coordinated primarily by an
interdisciplinary team within tertiary pediatric hospitals, aided by an outpatient PPC clinic and
must include a palliative home care service [51]. When these services are in place (but not instead),
a freestanding hospice and respite house represents an excellent addition (but not substitution) of the
services provided inpatient, in clinic, and at home. The World Health Organization (WHO) describes
that palliative care can be provided in tertiary care facilities, in outpatient clinics, in community health
and hospice centers, and in children’s homes [52].
A key component of family support, in addition to addressing the needs of the sick child [53],
must be geared toward the siblings [54,55] and parents/caregivers [56].
3.5. Myth 5: Patients and Their Parents Need to Make a Choice between “Fighting for a Cure” or “to Give
up Hope”
Even when there might not be a realistic hope for cure, pediatric patients with a serious illness
and their parents may opt for continued disease directed treatment [21]. This might be motivated by
the desire to extend life, to palliate symptoms related to progressive disease, or the hope for a miracle.

In discussions of treatment options with families, one might choose statements, as suggested by Wolfe,
such as “The very nature of miracles is that they are rare. However, we have seen miracles, and they
have occurred both on and off treatment” [57]. In other words, a child does not have to continue on
disease-directed therapy in order to preserve hope, especially when the therapy significantly impacts
the child’s remaining quality of life. In fact, a large adult study could demonstrate that chemotherapy
for end-stage cancer does not prolong life, however reduces the quality of life [58].
Caring for a child at end-of-life is emotionally very difficult. It may be particularly challenging
for clinicians and parents to consider the early integration of PPC because this may be perceived
as ‘giving up’. As a result, the emotional cost of the recognition that a child may die could impede
planning for optimal pain and symptom management and psycho-social-spiritual support. In fact,
the “hope for cure” and “pediatric palliative care” include and complement each other. PPC translates
into advanced management to maintain or improve quality of life and children can graduate from
PPC. No matter the treatment goals, despite the prevailing myth, disease-directed care and excellent
symptom relief must and can be provided simultaneously.
Date has shown that the integration of PPC explicitly does not result in giving up hope. Engaging
in advanced care planning increases the patients knowledge without diminishing hope, increasing
hopelessness, nor inducing anxiety in patients with advanced cancer [59]. The disclosure of a terminal
prognosis does not mean loss of patient hope: Instead, hope was redefined on a goal other than
cure [60]. In pediatrics, there is no evidence that prognostic disclosure makes parents less hopeful.

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Instead, the disclosure of a prognosis by physician can support hope, even when prognosis is poor [61].
Parents who are upset by prognostic information are no less likely to want it. The upsetting nature
of prognostic information does not diminish parents’ desire for such information, its importance to
decision making, or parents’ sense of hope [62].
During a goals of care discussion with a family of child with a serious illness, a question such as

“Considering what your child is up against, what are you hoping for?” may be posed by the clinician.
Not surprisingly, the response may be “Cure from the disease” or “A miracle”. The clinician may
then respond “I hope this too. Just in case the disease cannot be cured—What else are you hoping
for?” By exploring the extend of hope further, families may wish for very advanced treatment and
prevention of pain and distressing symptoms, the possibility to go home, for grandmother to visit,
to hold their child more often, or many other things. Blazin et al. in this special edition explore how to
translate evidence into practice and communicate effectively [30]. Even when the underlying condition
cannot be cured, PPC will never give up hope. Sometimes, it appears that the best chance for these
children to truly live is for their parents and treating clinicians to accept the fact that this child might
die [63]. Not surprisingly, data reveals, that religion, spirituality or life-philosophy play an important
role in the life of most parents whose children receiving PPC [64].
3.6. Myth 6: Administering Morphine Causes Respiratory Depression and Hastens Death
To paraphrase Sykes [65] “Morphine kills the pain, not the patient. A physician killing a patient
in name of pain or dyspnea relief is not merciful, just incompetent.” An enduring misconception is
the belief that in the management of pain and dyspnea, opioids will hasten death and should only
be administered as a last resort. This was contradicted in the adult literature [66] and our PPC teams
commonly observe that administering opioids and/or benzodiazepines, together with comfort care
to relieve dyspnea and pain, not only improves the child’s quality of life, but also prolongs life [67].
A retrospective cohort study (n = 223 adult oncologic patients) reviewing the mean survival in relation
to opioid use found less than a two-fold increase in their initial opioid dose resulted in 9 days survival,
and more than a two-fold increase in 22 days survival [68].
One of the most common sources of pain and distress in children is visceral hyperalgesia and
feeding intolerance, addressed by Hauer in this Special Issue [69].
3.7. Myth 7: PPC Teams Are Too Expensive
A pediatric palliative care team is now expected standard of practice in every children’s hospital.
Every single one of the “U.S. News & World Report honor roll children’s hospitals” have palliative
care teams [70]. Overwhelming evidence now demonstrates, that children’s hospitals in fact cannot
afford to not have a PPC team anymore. PPC team basically “grease the wheels” of a clinical institution,
such as reducing burnout and as a result staff turn-over among pediatric staff. For instance, in the
United States more than 30% of all new nurses elect either to change positions or leaving nursing

completely within the first 3 years of clinical practice [71]. Leading reasons include emotional distress
related to patient care. The average cost of turnover for a bedside nurse ranges from $36,900 to $57,300,
resulting in the average hospital losing $4.9 million–$7.6 million per year. Each percent reduction in
nursing turnover will cost/safe the average hospital an additional $379,500 [72]. PPC services can help
reducing turnover and improving overall job satisfaction and performance important in to assuring
patient and family satisfaction while promoting quality care.
In addition to providing advanced clinical care, pediatric palliative care services are also
cost-effective (although arguably not a single PPC team member worldwide provides clinical care
“to safe money” to the institutions or health insurers, but rather, because it provides superior clinical
care to children with advanced serious illnesses. PPC involvement results in better outcomes and
lower costs: Palliative care program reduces stress, costs of care for children with life-threatening
conditions [73–75]. A retrospective study of 425 children receiving pediatric palliative home care in
the US comparing pediatric hospital resource utilization before and after PPC enrollment decreased in

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non-cancer patients the length-of-hospital-stay by 38 days and decreased hospital charges $ 275,000
per patient [76].
4. Conclusions
Despite significant growth and advances over the last two decades, a large number of children’s
hospitals currently either do not have a PPC service, or it significantly under-resourced and
underfunded. When implementing a PPC service, the development often goes through four steps in
their development. These should be anticipated, and an action plan might include

•
•
•

•
•

Denial: Document unmet needs, undertake surveys among staff, patients and providers
Palliphobia: Close collaboration with colleagues; disciplined planning, rapid conflict resolution
Pallilalia: Documentation of PPC value to leadership; grand rounds by expert in field and
external review
Palliactive: “Do good & talk about it”, perform QI, evaluate value of PPC in ACO/bundled
payments environment; include clinical and administrative innovators in program development
Secure funding: “Make sure your passion is connected with somebody’s payment system”

High-quality pediatric palliative care for children with serious illnesses is now an expected standard
of medicine. However, even in resource-rich settings, there remain significant barriers to achieving
optimal care related to lack of formal education, reimbursement issues, the emotional impact of
caring for a dying child, and most importantly the lack of interdisciplinary PPC teams with sufficient
staffing. Whenever possible, treatment should focus on continued efforts to control the underlying
illness. At the same time, children and their families should have access to interdisciplinary care
aimed at promoting optimal physical, psychological and spiritual wellbeing. Persistent myths and
misconceptions have led to inadequate symptom control in children with life-limiting diseases.
Pediatric Palliative Care advocates the provision of comfort care, pain, and symptom management
concurrently with disease-directed treatments. Families no longer have to opt for one. They can pursue
both, and include integrative care to maximize the child’s quality of life.
Conflicts of Interest: The authors declare no conflict of interest.

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© 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access
article distributed under the terms and conditions of the Creative Commons Attribution
(CC BY) license ( />
13


children
Review

Pediatric Palliative Care in Infants and Neonates
Brian S. Carter 1,2,3
1
2
3

University of Missouri-Kansas City School of Medicine, 2411 Holmes Street, Kansas City, MO 64108, USA;
; Tel.: +1-816-701-5268; Fax: +1-816-302-9965
Children’s Mercy Hospital, Kansas City, MO 64108, USA
Division of Neonatology and Bioethics Center, 2401 Gillham Road, Kansas City, MO 64108, USA

Received: 7 December 2017; Accepted: 1 February 2018; Published: 7 February 2018

Abstract: The application of palliative and hospice care to newborns in the neonatal intensive care unit
(NICU) has been evident for over 30 years. This article addresses the history, current considerations,
and anticipated future needs for palliative and hospice care in the NICU, and is based on recent
literature review. Neonatologists have long managed the entirety of many newborns’ short lives,
given the relatively high mortality rates associated with prematurity and birth defects, but their ability
or willingness to comprehensively address of the continuum of interdisciplinary palliative, end of life,

and bereavement care has varied widely. While neonatology service capacity has grown worldwide
during this time, so has attention to pediatric palliative care generally, and neonatal-perinatal
palliative care specifically. Improvements have occurred in family-centered care, communication,
pain assessment and management, and bereavement. There remains a need to integrate palliative care
with intensive care rather than await its application solely at the terminal phase of a young infant’s
life—when s/he is imminently dying. Future considerations for applying neonatal palliative care
include its integration into fetal diagnostic management, the developing era of genomic medicine,
and expanding research into palliative care models and practices in the NICU.
Keywords: neonatal; palliative care; comfort care; pain

1. Introduction
The provision of palliative or hospice care for newborn infants was first introduced in the 1980s.
At the time, hospice principles were being disseminated in the US and their applicability to certain
newborns and young infants was noted by Whitfield et al. [1] and by Silverman [2]. Since 1982,
there has been increased growth in the field of palliative care in general, and in both pediatric and
neonatal palliative care specifically. Indeed, the specialty of palliative and hospice medicine has gained
recognition in North America and internationally with the attendant growth of training programs in
both the clinical and academic arenas.
2. Materials and Methods
A brief, but pertinent, review of the past 10 years’ clinical literature was conducted to examine
the breadth of neonatal palliative care as it is currently practiced. Throughout this process,
additional clinical needs, potential research ideas, and future considerations were determined and are
herein addressed.
3. Results
As the specialty of neonatology has grown around the world in the last three decades, the role
for palliative care (PC) has likewise been recognized in the neonatal intensive care unit (NICU) and
pediatric intensive care unit (ICU)—both common locales for neonatal and infant mortality [3–5].
Over the past 15 years, marked improvements have occurred around the circumstances in which
Children 2018, 5, 21; doi:10.3390/children5020021

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Children 2018, 5, 21

newborn and young infants die in North American hospitals. There has been attention drawn
to patient needs such as pain and symptom management [6,7], spiritual support [8], honoring
cultural practices [5], understanding grief [9], and employing a breadth of bereavement activities and
services [10,11].
Some particular features of providing PC for newborns and young infants include how pregnancy
is unique among human experiences and the veritable uniqueness of each pregnancy—and child—from
all others. Mothers and fathers generally enter into pregnancy with anticipation and hopefulness,
having a future-oriented idea of their yet to be born infant. That a newborn may be critically ill,
extremely premature, or born with significant birth anomalies that threaten his/her life and wellbeing
is never truly anticipated. When discovered—at birth or even beforehand with fetal diagnostics—the
pregnancy generally takes a dramatic turn as hopeful anticipation is replaced by fear, joy may be
eclipsed by guilt, and the experience of pregnancy and childbirth becomes medicalized—often with
obsessive thoughts surrounding each clinic visit, imaging study, or test. In addition to cure-oriented
and life-extending neonatal intensive care, the provision of concurrent PC may provide supportive
care for the patient and family, and may help in decision-making [12,13].
As is true in other areas of pediatric PC, neonatal PC has had a somewhat divided history. In the
NICU, there have long been a number of patients who have been treated for weeks to months only to
reach a plateau or stagnation in their progress toward growth and healing. They remain ventilated,
perhaps dependent upon intravenous nutrition, have endured infections and maybe bowel problems,
their livers are impacted by cholestasis, and they may even have parenteral nutrition-associated liver
disease. Some have endured brain injury. Each day their care is challenging, and on any given day they
might have an acute decompensation for which escalating support is required—often accompanied by
analgesia and/or sedation to minimize cardiopulmonary instability or neurologic agitation. These

infants may further decompensate or develop secondary pulmonary arterial hypertension along
with their bronchopulmonary dysplasia (BPD). Any given infection may take their life. For these
infants—some of whom may linger, and others may in fact be dying—neonatal PC may be an adjunctive
care paradigm that is added to their continued intensive care. For some, the cure-oriented care may
yield to a palliative paradigm after intensive counseling of parents, exploration of options with the
interdisciplinary neonatal team, and thoughtful reflection. Psychosocial support for the family is
essential and may attend to anticipatory grief; spiritual support may increase, and a decision to
limit ongoing life-support may result in a mutually agreed upon redirection of care towards comfort
with a reduction in vital-sign and invasive monitoring, phlebotomies, and imaging tests. In time,
a compassionate withdrawal of life-supportive technology is performed, and caregivers work with
families to orchestrate a meaningful time with extended family, rituals, and allow for the infant’s
passing. In such cases, a period of focused palliation culminates in veritable hospice care in the NICU,
typically associated with the withdrawal of life-sustaining medical treatments.
As neonatal palliative care has developed, and an expanding literature is at the disposal of
clinicians (Figure 1), there has been greater consideration for PC in the NICU. In recent years,
especially as prenatal diagnostics have improved and life-limiting conditions are diagnosed prenatally,
some newborn infants will receive concurrent palliative care (even beginning with conversations
before birth) while being cared for in the NICU [14–16]. The care may be oriented toward confirming a
prenatal diagnosis and exploring care options, or be directed at the baby’s comfort while not taking
on intensive or invasive technological care. In the latter situation, time with parents, human contact,
warmth, and symptom relief may be the predominant mode of support [17]. For others, such as
a newborn with hypoplastic left heart syndrome, there may be intensive care provided while the
neonatal, cardiology, and cardiovascular surgical specialists confer about palliative surgical options
or perhaps limits or confounders (such as prematurity, additional birth defects, or the severity of
anatomical size constraints that make surgery more difficult) [18]. When concurrent palliative care
is made available, the added value of an interdisciplinary team, psycho-social-spiritual support,
and expert pain and symptom management are acknowledged as enhancing the baby’s and family’s

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Children 2018, 5, 21

quality of life even while cure-oriented or disease-modifying treatments are offered. The focus is on
the quality of life and relationships while living with a life-limiting condition [19,20].

PUBMED NEONATAL PALLIATIVE
CARE CITATIONS: 1971 - 2017
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1971

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6
7
1980

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19

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12
12

11

20

26

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1990

20

25
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Year

15

30
31

19

31
24

2000

33

30

35

34
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43
59
60

2010

64
64
96

83

70
79

90
106

113
142

111
108

2017

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20

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60

80

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120

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160

Number of publications
Figure 1. Increasing publications addressing neonatal palliative care.

A working definition for neonatal palliative care can be found from the organization Together for
Short Lives, a children’s palliative care group in the UK:
“Palliative care for a fetus, neonate, or infant with a life-limiting condition is an active and
total approach to care, from the point of diagnosis or recognition, throughout the child’s life,

at the time of death and beyond. It embraces physical, emotional, social, and spiritual
elements and focuses on the enhancement of quality of life for the neonatal infant and
support for the family. It includes the management of distressing symptoms, the provision
of short breaks, and care through death and bereavement” [21].
Note here that this involves far more than care of the imminently dying newborn. Indeed,
the many domains of PC outlined by Ferrell in 2005 [22] are present and require consideration and
16